Primary Central Nervous System Vasculitis Mimicking Susac Syndrome and Multiple Sclerosis With Long-Term Remission and Spontaneous Resolution of Lesions: A Case Report.

Primary central nervous system vasculitis (PCNSV) is an angiitis localized to the central nervous system (CNS), with various manifestations and no specific biomarkers. Herein, we report a case of PCNSV that presented with an unusual course. A 40-year-old Japanese male developed inner ear symptoms and visual field disturbances. Later, at 42 years of age, the patient developed right hemiparesis and was diagnosed with multiple sclerosis (MS). He received methylprednisolone pulse therapy, which improved his symptoms and resolved most brain lesions. Subsequently, he did not visit the hospital for 13 years, during which time he experienced no relapse. At 55 years of age, he presented to our hospital with fatigue and dizziness. Susac syndrome was suspected because of sensorineural hearing loss and snowball lesions in the corpus callosum. Some of the brain lesions resolved spontaneously. A biopsy was performed on a right frontal lobe lesion, which revealed vasculitis with fibrinoid necrosis, no demyelinating lesions, no amyloid positivity, and no infiltration of atypical lymphocytes. With no evidence of vasculitis in other organs, the patient was diagnosed with PCNSV. The patient was treated with methylprednisolone pulse therapy, followed by oral prednisolone (1 mg/kg/day). The prednisolone was tapered off, and no relapse of symptoms or new lesions on magnetic resonance imaging (MRI) were noted. As observed in this case, even in a scenario suggestive of Susac syndrome or multiple sclerosis, PCNSV should be considered a differential diagnosis and confirmed via brain biopsy.

Effect of early dose increase of evocalcet for intractable hypercalcemia caused by parathyroid carcinoma.

Summary: Hypercalcemia due to parathyroid carcinoma (PC) is safely and quickly controlled with rapidly increasing evocalcet doses. Most parathyroid carcinomas are detected because of hypercalcemia due to primary hyperparathyroidism (PHPT). Hypercalcemia becomes more severe in patients with PC than those with parathyroid adenoma or hyperplasia. Hypercalcemia often causes renal dysfunction, gastrointestinal symptoms, and psychiatric symptoms. Consequently, the serum calcium level needs to be promptly corrected. Here, we report a case of PC with remarkably persistent hypercalcemia, which we safely and quickly controlled with rapidly increasing evocalcet doses. A 77-year-old female presented with renal dysfunction. Her serum calcium (Ca) and intact parathyroid hormone serum levels were 13.9 mg/dL and 1.074 pg/mL, respectively. Her renal function worsened because of hypercalcemia due to PHPT. Technetium-99 m methoxy-isobutyl-isonitrile parathyroid scintigraphic examination revealed an accumulation below the right thyroid lobe. CT examination showed a 35-mm mass. Hypercalcemia needed to be immediately corrected because of the patient's worsening renal function. Evocalcet treatment at a gradually increasing dose of up to 20 mg over 3 weeks allowed her serum Ca level to be maintained below 11 mg/dL. Only mild nausea was observed at the beginning of the treatment. The mass was suspected as PC because the hypercalcemia was refractory to high-dose evocalcet. The patient was treated with parathyroidectomy and ipsilateral thyroidectomy. PC was diagnosed based on the pathological findings of capsular and venous invasion. The patient's renal function improved and surgery could be safely performed by promptly correcting hypercalcemia. Learning points: Hypercalcemia due to parathyroid carcinoma (PC) is often more severe than that caused by parathyroid adenoma or hyperplasia. PC is a rare disease, but it should be considered if the patient has intractable hypercalcemia due to primary hyperparathyroidism (PHPT). Evocalcet, which is used to treat hypercalcemia due to PHPT, does not interact with P450 (CYP) and causes few side effects. Complications, including renal dysfunction, were improved and the surgery could be safely performed by promptly correcting hypercalcemia. PC has a high recurrence rate. En-block excision is necessary when PC is suspected.

Primary signet-ring cell carcinoma of the renal pelvis: An autopsy case.

Introduction: Signet-ring cell carcinoma is an extremely rare histological variant of upper urinary tract carcinoma, associated with poor prognosis. Case presentation: We report a case of a 75-year-old female diagnosed with left primary upper urinary tract signet-ring cell carcinoma, initially treated with surgery. Post-surgical development of multifocal metastases was followed by a devastating clinical course. An autopsy confirmed the tumor's primary origin in the upper urinary tract. Conclusion: We experienced a case of upper urinary tract signet-ring cell carcinoma, with a rare opportunity to thoroughly confirm its primary site with an autopsy.

Long-term response on letrozole for gastric cancer: A case report.

RATIONALE: Hormone therapies, particularly those targeting estrogen and its receptors, are a key treatment modality for patients with estrogen receptor (ER)-positive breast or ovarian cancer. Some gastric cancers (GCs) express ERs, and preclinical studies suggest the potential of estrogen-targeting hormone therapy on GC; however, the clinical relevance of this hormone therapy on GC treatment has not been well elucidated. PATIENT CONCERNS: An 80-year-old female was admitted to our department with hypogastric pain and vomiting. Computed tomography demonstrated small bowel obstruction, and laparotomy after bowel decompression revealed peritoneal dissemination consisting of a poorly-differentiated adenocarcinoma. Intestinal bypass between the ileum and transverse colon was performed. DIAGNOSES: The tumor was ER- and mammaglobin-positive, indicating that it originated from a breast cancer. Diagnostic imaging revealed no evidence of breast cancer; however, right axillary ER- and mammaglobin-positive lymphadenopathy was found. INTERVENTIONS: The patient received hormone therapy using letrozole based on a clinical diagnosis of occult breast cancer with peritoneal dissemination and right axillary lymph node metastasis. OUTCOMES: The patient remained disease free until 37 months but deceased at 53 months from the onset of disease. An autopsy revealed no tumor cells in the right breast tissue; however, there was a massive invasion of cancer cells in the stomach. LESSONS: A patient with ER positive GC with peritoneal dissemination and right axillary lymph node metastasis presented remarkable response to letrozole. The long-term survival obtained using letrozole for a patient with GC with distant metastasis suggests the potential of estrogen targeting hormone therapies for GC.

A case of unique endoscopic findings of intestinal endometriosis exposed to the mucosa: aggregation of papillary protruded bulges from the submucosal elevation of the rectum.

Intestinal endometriosis exposed to the mucosa is relatively rare. Therefore, its endoscopic findings with pit pattern and magnifying endoscopy with narrow-band imaging and clinicopathological features of intestinal endometriosis exposed to the mucosa have not been well documented until now. A 44-year-old woman was suspected to have gastrointestinal bleeding by positive fecal occult blood test. Colonoscopy revealed a hemicircular submucosal tumor whose surface was covered with easy-bleeding papillary bulges in the rectum. Pit pattern analysis and magnifying endoscopy with narrow-band imaging revealed straight microvessels among the straight pits arranged in a radial manner, and the avascular area with no pit pattern of the top of the bulge. These findings were different from those of polyps or cancer. Biopsy specimens from the protruded lesions were diagnosed as rectal mucosal endometriosis by hematoxylin-eosin staining and immunohistochemical examination. Surgical resection was suggested to the patient, but the patient did not favor surgical treatment. After the diagnosis dienogest treatment started and successfully relieved her abdominal pain. Malignant transformation of the endometriotic lesion has not arisen to this date.

A case of pulmonary histoplasmosis diagnosed after lung lobectomy.

BACKGROUND: Histoplasmosis is considered a fairly rare imported mycosis in Japan. Here we report a case of histoplasmosis describing the preoperative findings, histopathological findings, supposed infection route, and appropriate treatment, including the postoperative management. CASE PRESENTATION: A healthy 65-year-old man was found at routine medical check-up to have an abnormal opacity on chest radiography. A chest computed tomography (CT) scan showed a nodular lesion in the posterior basal segment of the right lung, as well as two smaller nodules in the same lobe. This was highly suggestive of primary lung cancer with pulmonary metastases in the same lobe. We thus performed a right lower lobectomy with hilar and mediastinal lymph node dissection via thoracotomy. The lesions were diagnosed as pulmonary histoplasmosis on histopathology. At 6-month follow-up examination, the patient was free from fungal infection without any postoperative medication. CONCLUSIONS: We describe a patient with pulmonary histoplasmosis diagnosed following surgical lobectomy. The possibility of pulmonary histoplasmosis should be considered in the differential diagnosis of pulmonary nodular lesions.

"Pure" Suprasellar Schwannoma Presented with Communicating Hydrocephalus: A Case Report.

Schwannoma is a benign peripheral nerve sheath tumor originating from Schwann cells. Most intracranial schwannomas arise from vestibular nerve and schwannoma in the suprasellar region is extremely rare. A 64-year-old man presented with walking disturbance and blurred vision for three months. Lateral hemianopsia in the left eye and brachybasia were observed. Magnetic resonance imaging revealed a suprasellar tumor with strong contrast enhancement associated with communicating hydrocephalus. The cerebrospinal fluid tap test improved gait disturbance. Hypothalamic stimulation test revealed hypo-reaction of GH, FSH and LH. After ventriculo-peritoneal shunting, the tumor was totally removed via a bilateral front-basal approach with a clinical diagnosis of craniopharyngioma. No adhesion was observed between the tumor and surrounding structures such as meninges and brain. The histopathological diagnosis was schwannoma. Here we report a case of suprasellar schwannoma associated with communicating hydrocephalus that has not ever been previously reported, with special reference to its pathogenesis.

The Resection of Thyroid Cancer Was Associated with the Resolution of Hyporesponsiveness to an Erythropoiesis-stimulating Agent in a Hemodialysis Patient with Aceruloplasminemia.

We herein report the case of a hemodialysis patient whose response to an erythropoiesis-stimulating agent (ESA) improved following the resection of thyroid cancer. Her hemoglobin level remained below 7 g/dL, despite the use of ESA. During the search for the causes of her hyporesponsiveness to ESA, papillary thyroid cancer and aceruloplasminemia were found. The existence of other potential causes, such as iron deficiency, infectious disease, severe hyperparathyroidism and malnutrition were ruled out. Following the resection of the thyroid cancer tumor, her hemoglobin level increased to 10.2 g/dL over a period of 4 months. This is the first report to demonstrate the resolution of hyporesponsiveness to ESA following the resection of a malignant tumor.

Poorly Differentiated Gastric Adenocarcinoma Can Mimic Hilar Cholangiocarcinoma.

This report describes two cases with obstructive jaundice caused by poorly differentiated gastric adenocarcinoma. Computed tomography scans showed circumferential stenosis in the hilar bile ducts. Endoscopic retrograde cholangiopancreatography showed dilatation of the bilateral hepatic ducts and stenosis of the common hepatic ducts from the bifurcation of the bilateral hepatic ducts. The first diagnoses were hilar cholangiocarcinoma and biliary drainage decreased serum bilirubin; however, both patients died of cancer within a short period of time. Autopsies revealed lymphatic vessel invasion and possible subepithelial invasion by gastric adenocarcinoma into the hilar bile ducts. A differential diagnosis should thus be required in suspected cases of hilar cholangiocarcinoma.

A case of primary adrenal diffuse large B cell lymphoma presenting with severe hyponatremia.

We herein report a case of primary adrenal lymphoma with severe hyponatremia. The patient was admitted for an evaluation of severe hyponatremia and an enlarged bilateral adrenal mass, which were found in a previous examination for causes of general fatigue and anorexia. Laboratory data, including the serum levels of sodium (115 mEq/L), osmolality (239 mOsm/kgH2O), ADH (5.8 pg/mL), cortisol (11.6 µg/dL), free T3 (2.42 pg/mL), urinary Na (117 mEq/L) and urine osmolality (490 mOsm/kgH2O), fulfilled the diagnostic criteria for the syndrome of inappropriate secretion of ADH (SIADH). An abdominal computed tomography scan revealed a large bilateral adrenal mass. A biopsy of the enlarged left adrenal mass revealed diffuse large B cell lymphoma, which was negative for ADH protein. Hydrocortisone treatment normalized the patient's body temperature and serum sodium concentration. In this case, hyponatremia developed when both adrenal glands were involved and was normalized with hydrocortisone. These findings suggest that adrenal insufficiency was the cause of hyponatremia, although the basal serum cortisol was normal. The current case suggests that the administration of hydrocortisone is recommended if suspicious clinical signs or symptoms are found in severe hyponatremia, even if hyponatremia is associated with a normal serum cortisol level and fulfills the diagnostic criteria for SIADH.

[A case of intravascular large B-cell lymphoma that presented with recurrent multiple cerebral infarctions and followed an indolent course].

A 66-year-old woman presented with vertigo and deafness. Diffusion-weighted magnetic resonance imaging of the head showed multiple cerebral infarctions involving several blood vessel regions. A diagnosis of cardiogenic embolism was made, and anticoagulation therapy was begun. The woman had no additional symptoms until suddenly developing left hemiparesis one year later. She was again found to have multiple cerebral infarctions. The hemiparesis gradually improved, but ataxic gait and apraxia appeared and progressed over two weeks. Holter ECG, carotid ultrasound, and transthoracic/transesophageal echocardiography revealed no evidence of cardiogenic embolism. However, serum lactate dehydrogenase (LDH) and soluble interleukin-2 receptor (sIL2R) levels were elevated (LDH, 782 IU/l; sIL2R, 1,396 IU/ml), which suggested malignant lymphoma. Contrast chest/abdominal CT scan and gallium-67 scintigraphy revealed no evident lesions; however, random skin biopsy and open brain biopsy showed that blood vessels were infiltrated by CD20-positive atypical lymphocytes. These findings were consistent with intravascular large B-cell lymphoma. This type of lymphoma is known as a rapidly progressive disease with poor prognosis, but this case followed an indolent course, with a one-year interruption in disease progression.

[IgG4-related kidney disease: a long-term follow up case of pseudotumor of the renal pelvis].

A 69-year-old man had undergone left ureteronephrectomy because of a left renal pelvic tumor, however the pathological diagnosis was inflammatory pseudotumor. About 1 year later, computed tomography showed a mass at the right kidney near the hilar. Ureterorenoscopy and urine cytology were performed, and their results showed no evidence of malignancy. He had been followed closely without therapy. The mass increased in size during follow-up, and we reviewed the surgical specimen of the left ureteronephrectomy. Immunohistochemical studies revealed diffuse infiltration by IgG4 positive plasma cell. His serum IgG4 was high. We diagnosed him as IgG4-related kidney disease. In response to treatment with corticosteroid, the size of the tumor and serum IgG4 levels decreased. Most reported cases of IgG4-related disease involving kidney have a history of prior pancreatic involvement. We report a rare long term follow-up case of IgG4-related kidney disease without pancreatic involvement.

Superior mediastinal paraganglioma associated with von Hippel-Lindau syndrome: report of a case.

Extra-adrenal pheochromocytomas are termed paragangliomas. Paragangliomas in the mediastinum, especially the superior mediastinum, are extremely rare. It is known that paragangliomas or pheochromocytomas occur in combination with von Hippel-Lindau syndrome. We present the case of a non-functional superior mediastinal paraganglioma in a patient with von Hippel-Lindau syndrome, without a familial history suggestive of the condition. This case highlights that we should be aware of possible sporadic von Hippel-Lindau syndrome in patients with a mediastinal paraganglioma.

Polyarteritis nodosa diagnosed by surgically resected jejunal necrosis following acute abdomen.

The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.

Enhanced chemiluminescent detection scheme for trace vapor sensing in pneumatically-tuned hollow core photonic bandgap fibers.

We demonstrate an in-fiber gas phase chemical detection architecture in which a chemiluminescent (CL) reaction is spatially and spectrally matched to the core modes of hollow photonic bandgap (PBG) fibers in order to enhance detection efficiency. A peroxide-sensitive CL material is annularly shaped and centered within the fiber's hollow core, thereby increasing the overlap between the emission intensity and the intensity distribution of the low-loss fiber modes. This configuration improves the sensitivity by 0.9 dB/cm compared to coating the material directly on the inner fiber surface, where coupling to both higher loss core modes and cladding modes is enhanced. By integrating the former configuration with a custom-built optofluidic system designed for concomitant controlled vapor delivery and emission measurement, we achieve a limit-of-detection of 100 parts per billion (ppb) for hydrogen peroxide vapor. The PBG fibers are produced by a new fabrication method whereby external gas pressure is used as a control knob to actively tune the transmission bandgaps through the entire visible range during the thermal drawing process.

Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4.

The possible involvement of immunoglobulin G4 (IgG4) in the pathogenesis of idiopathic sclerosing lesions has been suggested. In this study, a clinicopathologic analysis was performed to reveal characteristics of retroperitoneal fibrosis relating to IgG4. The study involved 17 patients with retroperitoneal fibrosis. Immunohistochemistry revealed numerous IgG4-positive plasma cell infiltrates in 10 cases (IgG4-related), but only a few positive cells in 7 cases (non-IgG4-related). All patients with IgG4-related retroperitoneal fibrosis were male, whereas all except 1 with unrelated lesions were female. Histologically, eosinophilic infiltration (>5 cells per high-power field) and obliterative phlebitis were commonly observed in IgG4-related lesions. Serologically, serum IgG and IgG4 concentrations were significantly higher in the IgG4-related cases, with the IgG4 concentrations all over 135 mg/dL (the upper limit of the normal range). Steroid therapy was performed in 13 cases, and was effective irrespective of IgG4. Three patients had recurrence during the follow up. Five of 10 IgG4-related cases had sclerosing lesions at other sites. The only tests that reliably distinguish the 2 groups were serum IgG4 levels or IgG4/IgG ratio in the plasma cells in a tissue biopsy. The only major clinical difference was the striking male predominance in IgG4-related cases. In conclusion, this study revealed that retroperitoneal fibrosis could be classified as IgG4-related or not. This distinction seems important to help better characterize the biology/pathogenesis of both groups and better predict the possibility of other IgG4-related processes at other anatomic sites.

Histological differences between invasive ductal carcinoma with a large central acellular zone and matrix-producing carcinoma of the breast.

Carcinoma with a large central acellular zone (central acellular carcinoma, CAC) and matrix-producing carcinoma (MPC) have been recently noted as basal-like-type breast cancers, but the two entities are often confused. To clarify their histological differences, the histopathological sections of 15 CAC and seven MPC were examined and the following features were compared by reviewing slides: (i) mode of invasion; (ii) alteration of cancer cell adhesion in the transitional area between cellular and acellular zones; (iii) staining of the stromal matrix; (iv) lymphocyte infiltration; and (v) tumor grade. Complete agreement was required between two observers for the assessments of these features. All CAC had relatively sharp margins but showed infiltrative growth accompanied by eosinophilic intercellular matrix. In CAC there was abrupt transition between peripheral cellular and central acellular zones without alteration of cancer cell adhesion. In contrast, all MPC showed expansive growth with a well circumscribed margin, accompanied by basophilic and myxoid intercellular matrix. In MPC there was gradual transition from cellular to acellular areas with gradual loss of cancer cell adhesion. Histological grade 3 and peripheral lymphocyte infiltration were common features. It is suggested that CAC and MPC are histologically distinct entities, and that the aforementioned features are helpful for differential diagnosis.

Genetic diversity of two selected antigen loci in Babesia gibsoni Asian genotype obtained from Japan and Jeju island of South Korea.

Previous reports have shown that the secreted antigen 1 of Babesia gibsoni (BgSA1) and the thrombospondin-related adhesive protein of B. gibsoni (BgTRAP) are promising diagnostic reagents and vaccine candidates. Therefore, we determined the extent of nucleotide sequence variation in the BgSA1 and BgTRAP genes, obtained from eight isolates of B. gibsoni got from clinically infected dogs in geographically distinct areas of Japan and one isolate from Jeju island of South Korea. Sequence analyses have revealed that nucleotide diversity is lower in BgSA1 than that in BgTRAP. The mean number of non-synonymous (dn) nucleotide substitutions was significantly greater than that of synonymous (ds) ones per site in region II of BgTRAP. Overall, the results predict more allele-specific immunity to BgTRAP than that to BgSA1, which could be useful in designing and testing efficacy of diagnostic reagents as well as vaccine candidates for the B. gibsoni isolates from Japan and Jeju island of South Korea.

Cartilaginous features in matrix-producing carcinoma of the breast: four cases report with histochemical and immunohistochemical analysis of matrix molecules.

Matrix-producing carcinoma of the breast is a well-established entity in the group of metaplastic carcinoma, which is histologically characterized by myxochondroid matrix formation and is extremely rare. We describe here four additional cases of matrix-producing carcinoma of the breast. All cases of matrix-producing carcinoma show nest-like, sheet-like, and cord-like growth of tumor cells with cellular atypia, in addition to scattered cancer cells within myxoid or myxohyalinous stroma. Three of four cases showed an acellular or oligocellular matrix-rich zone in the center of the tumor. Immunohistochemically, cancer cells of all cases were positive for cytokeratins and epithelial membrane antigens and partially positive for sox9 and p63. Aggrecan and type II collagen, which are cartilage-specific matrix molecules, were deposited in the stroma of all cases. Type I and type IV collagens were also deposited on the stroma of all cases. These findings suggest that, although cancer cells of matrix-producing carcinoma of the breast are epithelial, they transdifferentiate to chondrocyte-like cells and produce cartilage-specific matrix molecules, which are useful markers for diagnosing matrix-producing carcinoma.

[Pericardial effusion due to metastatic prostate cancer: a case report].

We describe a patient whose complaints were related to pericardial effusion due to prostatic carcinoma. An 80-year-old man was admitted to our hospital because of chest discomfort and dyspnea. The chest radiograph revealed cardiomegaly and computed tomographic scan showed a large pericardial effusion. Pericardiocentesis revealed sanguinous exudates. Cytologic study suggested metastatic adenocarcinoma or malignant mesothelioma. He died suddenly because of ventricular tachycardia. At autopsy, the major finding was poorly differentiated adenocarcinoma of the prostate with metastases to the mediastinum.